Presentation
At present, only eleven cases have been reported. [ncbi.nlm.nih.gov]
Entire Body System
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Pathologist
We provide the largest case series to date and highlight the need for pathologists to have a high level of suspicion for PCH in patients with CTD. [ncbi.nlm.nih.gov]
Pathologists will find this book tremendously helpful when faced with difficult cases of interstitial lung disease or lung granulomas. It should be part of every surgical pathologist's library. [books.google.de]
The diagnosis can be made when pathologists see capillary overgrowth in a lung biopsy specimen. [phassociation.org]
Respiratoric
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Recurrent Chest Pain
Chest pain: may occur Treatment In histologically-proven [[Pulmonary Veno-Occlusive Disease]] (with or without pulmonary capillary hemangiomatosis) In response to pulmonary vasodilator therapy (mainly continuous intravenous epoprostenol, but also with [mdnxs.com]
Psychiatrical
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Suggestibility
The cause of the venous obliteration was not identified but the occasional identification of phlebitis suggests this plays a role in venous damage in some cases. [ncbi.nlm.nih.gov]
Urogenital
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Cryptorchidism
We report a case of an 18-year-old high school student, with a past history of cryptorchidism, presenting with recurrent pneumothorax. Routine laboratory findings were unremarkable. [ncbi.nlm.nih.gov]
Workup
The workup of a patient with suspected PAH is designed to confirm the diagnosis, exclude or confirm the presence of an underlying cause, and assess the severity of the disease. [clevelandclinicmeded.com]
X-Ray
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Kerley A Lines
There were no Kerley-B lines or pleural effusion. The ECG showed right axis deviation and right ventricular hypertrophy with ST–T changes ( Figure 2 ). [circ.ahajournals.org]
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Kerley B Lines
There were no Kerley-B lines or pleural effusion. The ECG showed right axis deviation and right ventricular hypertrophy with ST–T changes ( Figure 2 ). [circ.ahajournals.org]
Other Pathologies
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Lymphocytic Infiltrate
Cases with PCH also all showed a mild interstitial lymphocytic infiltrate but there was no venulitis or infarction. [ncbi.nlm.nih.gov]
Treatment
We describe a case of PH associated with limited SSc, where treatment with prostacyclin was complicated by pulmonary edema. Autopsy revealed PCH as the pathologic basis for the PH. [ncbi.nlm.nih.gov]
Prognosis
Diagnosis of PCH is challenging in the setting of CDH and portends a poor prognosis. [ncbi.nlm.nih.gov]
Disease (PVOD) Primary Pulmonary Hypertension (PPH) Chronic Pulmonary Thromboemboli Pathology Some evidence that proliferation of thin-walled capillaries in PCH is histologic reaction to PVOD Clinical Issues Normal pulmonary capillary wedge pressure Prognosis [radiologykey.com]
Prognosis is poor, with an estimated mean survival of 36 months. 1 A 53-year old man, former smoker (39 pack-years), presented with worsening dyspnoea upon exertion and fatigue lasting 4 years. [archbronconeumol.org]
Etiology
We herein present a case of PCH diagnosed after lung transplantation with a focus on its etiology and a key to clinical diagnosis. [ncbi.nlm.nih.gov]
Unlike most pathology textbooks, diseases are grouped on the basis of pathologic findings rather than etiology, mirroring the actual practice of surgical pathology. [books.google.de]
[…] rare cases without recognizable causative background disease ( Am J Surg Pathol 2006;30:850 ) Epidemiology Very rare, mostly in adults Mean age 30 years, range 2-71 years Pathophysiology Chronic passive congestion may lead to capillary proliferation Etiology [pathologyoutlines.com]
Epidemiology
Articles include: Epidemiology of Pulmonary Arterial Hypertension, Pathology of Pulmonary Hypertension, Genetics of Pulmonary Arterial Hypertension, Diagnosis of Pulmonary Arterial Hypertension, Pulmonary Hypertension Owing to Left Heart Disease, Pulmonary [books.google.de]
Consider diagnosis of "secondary PCH" or PCH-like changes for proliferations seen in association with other disorders, reserving "primary PCH" for extremely rare cases without recognizable causative background disease ( Am J Surg Pathol 2006;30:850 ) Epidemiology [pathologyoutlines.com]
Epidemiology Rare disorder Occurs mainly in children and young adults (12 reported cases) [[Pulmonary Veno-Occlusive Disease]], pulmonary capillary hemangiomatosis, and [[Idiopathic Pulmonary Arterial Hypertension]] may all represent various points on [mdnxs.com]
Epidemiology. Prognosis. Referral centre. Abstract Recently, our view of pulmonary hypertension has been changed by the significant progress made in understanding the pathobiology, epidemiology and prognosis of the disease. [revespcardiol.org]
Pathophysiology
[…] seen in association with other disorders, reserving "primary PCH" for extremely rare cases without recognizable causative background disease ( Am J Surg Pathol 2006;30:850 ) Epidemiology Very rare, mostly in adults Mean age 30 years, range 2-71 years Pathophysiology [pathologyoutlines.com]
The pathophysiological basis of the increase in pulmonary vascular resistance is hypertensive vascular disease in small arteries and pulmonary arterioles. [revespcardiol.org]
Pulmonary hypertension: Pathophysiology and clinical disorders. Baum GL, Crapo JD, Celli BR, Karlinsky JB, eds. Textbook of Pulmonary Diseases. Philadelphia, Pa: Lippincott-Raven; 1998. 1273-95. Wagenvoort CA. [emedicine.medscape.com]
Although the etiology and pathophysiology of PAH and PVH are distinct, they are similar in that both cause elevated PA pressure that inevitably leads to right ventricular (RV) dilation and remodeling, followed by RV failure and death if RV compensation [clevelandclinicmeded.com]
Prevention
Also very rarely they will continue to grow and eventually rupture....These growing hemangiomas become cavernous and may require surgery to prevent unwanted rupture. ...Read more See 1 more doctor answer 10 Is there collagen in capillary blood vessels [healthtap.com]
Diuretics - Usually oral tablets taken daily to prevent fluid retention. Atrial Septostomy - A hole is made between the right and left atriums in the heart the procedure is similar to having a right heart catheter done. [nzpah.org.nz]
While diuresis is indicated to prevent volume overload in most patients with PAH, diuretics should be used with caution. [clevelandclinicmeded.com]
Identification of GCN2 as new redox regulator for oxidative stress prevention in vivo. Biochem. Biophys. Res. Commun. 415, 120–124 (2011). 19. Fessel, J.P. et al. [nature.com]